Median Arcuate Ligament Syndrome – Literature Study and Osteopathic Considerations Luc Peeters

Introduction Figure 1 – Median arcuate ligament Median Arcuate Ligament Syndrome (MALS) is also known as coeliac trunk compression syndrome or Dunbar syndrome (Dunbar et al., 1965). Occasionally it is referred to as abdominal angina. The median arcuate ligament can compress the coeliac trunk. This occurs frequently but it is not understood why some cases are symptomatic while some remain asymptomatic. Various symptoms have been attributed to this syndrome, ranging from abdominal symptoms to musculoskeletal symptoms to psychological symptoms (Carey et al., 1969; Williams et al., 1985). The coeliac trunk was found where the median arcuate ligament crosses the midline. Classical medicine will often advise operative intervention (the ligament is cut) (Barcourt et al 1988, Ghosn et al., 1982; In 12 of 83 cadavers (14.46%) the coeliac trunk was further Mihas et al., 1977) but a clear indication for such surgery is from the ligament, in 35 cadavers (42.17%) the trunk touched the not present. Recently, laparoscopy has been used to relieve the ligament and in 36 cases (43.37%) the ligament crossed over the compression (Desmond et Roberts, 2005). The results of these trunk. The average distance between both structures was 0.94 cm operations are highly variable. and when overlapping this distance became 0.42 cm (Dunbar et al .,1965; Petrella et al., 2006). Certain authors suggest that the In the osteopathic practice patients with vague abdominal coeliac trunk is in fact intrathoracic in some cases (Fadhli, 1968; and subdiaphragmatic pain - associated with eating or not - Warter et al., 1970), which leads to a congenital version of the are frequently encountered. The osteopath will often treat the compression syndrome. stomach function, the diaphragm and the segments of the upper digestive system. These patients often present with a very rigid Figure 2 – Coeliac trunk and flattened thoracolumbar spine. Decoaptation in this region will often improve the complaint. Many of these complaints could be associated with median arcuate ligament syndrome. Resultant ischemia of the organs in the upper digestive system and even ischemia of the mesenteric root could also be the part of the problem. As well as the arterial compression an irritation of the coeliac plexus could be contributing to the complaint. Anatomy Median arcuate ligament Relative to the spine the coeliac trunk is at the level of The median arcuate ligament is the tendinous junction T12-L1. between the right and left crus of the diaphragm (Balaban, 1997; Horton et al., 2005). It is the anterosuperior boundary of the aortic hiatus. The histology of the ligament demonstrates dense connective tissue infiltrated by fat cells, blood vessels and nerves. Striated muscle fibres are present together with collagen fibres.

Page 14 The AAO Journal Volume 20, Issue 2, june 2010 Figure 3 – Topography of the coeliac trunk Pathology Arterial compression Due to compression of the coeliac trunk the arterial circulation of various digestive organs is also likely to be compromised. Anatomically, it is most likely that the arterial supply of the stomach, spleen and liver are the most affected. During exhalation the compression is increased due to the fact that the trunk ascends (Reuter, 1971; Reuter et Bernstein, 1973). This is equally so in case of a diaphragm high position dysfunction – due to fascial retractions in the thorax, congestion of subdiaphragmatic organs or muscular weakness. Figure 5 – Distribution of the arterial supply to the digestive organs Figure 4 – Compression of the coeliac trunk

The coeliac trunk is also the origin of the suspensory muscle of the duodenum. The suspensory muscle of the duodenum is the bond between duodenum III and IV and the coeliac trunk. This Figure 6 – Arterial supply to the stomach muscle consists of smooth muscle and connective tissue (Saenco et al., 1989). Coeliac plexus The majority of the abdominal sympathetic nervous tissue is found anterior and lateral upon the abdominal aorta. The upper part of this abdominal sympathetic tissue is known as the coeliac plexus. The coeliac plexus is found at the level of T12-L1 (Thomson et al., 1977). Studies of the individual variability of the location of the coeliac plexus show that in 32% of cases the coeliac plexus was an accumulation of numerous smaller ganglia linked by a network of nerve fibres, relatively removed from the blood vessels branching from the aorta. In 38% of case the coeliac plexus was a grouping of medium sized ganglia concentrated around the coeliac trunk, mostly on the left side up to the Via compression from the ligament ischemia can develop branching of the superior mesenteric a. and without contact with in the organs of the digestive system. Such ischemia can be the median arcuate ligament. In 30% of cases the coeliac plexus mild but in serious cases can result in necrosis. In serious cases was a grouping of 2 to 4 ganglia inserting into the median arcuate where the symptoms are confirmed with arteriography, urgent ligament and forming a dense ring around the blood vessels surgery is required as this situation can be life threatening, most (Vlasova, 2000). certainly if combined with arteriosclerosis of the involved vessels The coeliac plexus is highly vascular (Promwikorn et al., (Sulkowski et Wolters, 2000). 1988). The arterial blood comes from the aorta, inferior phrenic In such serious cases the ischemia becomes constant and leads a. and the suprarenal arteries. The venous drainage is via the to the symptoms being independent of eating. In more mild cases inferior phrenic v. and the vena cava inferior. the ischemic pain will most commonly occur 15 minutes after eating due to the fact that the arterial flow to the stomach cannot increase as required.

Volume 20, Issue 2, June 2010 The AAO Journal Page 15 Irritation of the coeliac plexus Functional osteopathic treatment Balaban et al showed in 1997 that surgical reduction of the The osteopath must be aware of this syndrome. Treatment pressure upon the coeliac trunk affected the contractile rhythm of of the serious, structural cases with associated arteriosclerosis the stomach. is contraindicated but during the functional stage osteopathic Gastroparesis and gastric arrhythmia were diagnosed pre- treatment is recommended. operatively and were shown to be resolved post-operatively by Before osteopathic treatment commences arteriosclerosis must way of a normal myoelectric activity of the stomach (of 3 cycles be discounted by appropriate testing. per minute). This finding indicates that the symptoms can be of neurogenic origin via irritation of the coeliac plexus. The osteopathic treatment has the following aims: Trophic changes to the coeliac plexus could be the underlying • Improving the mobility of the thoracolumbar junction: cause of the coeliac trunk stenosis (Drapanas et Bron, 1966; most important is improvement of the mobility into Harjola, 1963 & 1968; Harjola et Lahtiharju, 1968; Jamieson, flexion via a thoracolumbar decoaptation technique. 1986; Marable et al., 1966; Of Gossun et al., 1984). • The normalisation of the diaphragm function: most Dysfunction of the duodenojejunal angle important is increasing the inhalation capacity as it is during exhalation (or a dysfunction in high position of This functional sphincter has no actual sphincter fibres but the diaphragm) when the coeliac trunk ascends and the does suspend from the coeliac trunk and duo III and IV by way of chance of compression is most significant. a system consisting of smooth muscle (suspensory muscle of the duodenum), a striated muscle originating from the diaphragm and • The decongestion and stretching of the region ventral to fibrous tissue. It acts to limit the transit so that the function of the T12-L1: the region of the coeliac plexus is the aim here. duodenum is better controlled. If the functional sphincter system A fat-reducing diet is necessary for obese patients: the median does not properly function then the evacuation of the duodenum arcuate ligament consists of fat cells and any increase will is no longer exponential (first adequate filling must occur before increase the chance of compression. the contents are allowed to advance further). The stomach- duodenal evacuation is interrupted. Specific techniques When transit is too fast the result is poor digestion. This article is written for professional osteopaths. It is assumed therefore that all the necessary techniques required Symptoms to achieve the aims above are able to be carried out in safety. However, several specific techniques for this particular complaint The symptoms indicative of this syndrome are: are presented below. • Abdominal pain, not related to eating but due to the Active mobilization of the coeliac trunk ischemia (in 71% of cases). The patient is sitting on their knees and bent forward as far • Systolic murmur (in 15% of cases). as possible so that the abdomen approaches or even touched the • Nausea (in 29% of cases). knees. The patient abdominally inhales as deeply as possible and is instructed to hold their breath while actively flexing their • Lower thoracic and thoracolumbar pain (in 22% of cases). thoracolumbar region (kyphosis). In this way the increase in • Acidic reflux (in 17% of cases). abdominal pressure will flex the thoracolumbar region and the coeliac trunk will descend. The contraction of the diaphragm • Weight loss (in 15% of cases). The weight loss is due to during the inhalation will place cranial traction upon the median the lack of normal increase in blood supply 15 min after arcuate ligament. At the same time a drainage effect will occur in beginning to eat. The pain is usually associated with the abdominal venous system. eating. This technique is repeated several times. • Vomiting (in 15% of cases). Figure 7 – Active mobilization of the coeliac trunk • Diarrhoea (in 14% of cases). Occurs due to dysfunction of the coeliac plexus as is observed secondary to coeliac plexus block during pain therapy. • Breathlessness (in 14% of cases). • Murmur during auscultation due to the post-stenotic turbulence (Edwards et al 1970). The syndrome occurs most commonly in adults but has been described in children (Dubbins et Scholbach, 2006) most likely due to abnormal congenital anatomy.

Page 16 The AAO Journal Volume 20, Issue 2, june 2010 Defibrosing of the coeliac plexus (patient standing) Figure 9 – Defibrosing of the coeliac plexus (patient supine)

Figure 8 – Defibrosing of the coeliac plexus (patient standing) Stretch and mobilization of the region will create a defibrosing action for the coeliac plexus. Using the palmar side of three fingers, the osteopath contacts Lift and mobilization of the duodenojejunal angle the zone of the coeliac plexus. The patient is sitting and the osteopath stands behind the patient. While exahaling, the patient bends forward and Using the fingers of both hands the osteopath hooks into the the osteopath strengthens the abdomen just left of the midline, at the height of the navel, under contact to cranial/posterior/ the duodenojejunal angle. This structure is not palpable as such right, in the direction of the but the anatomical location is used. vertebral bodies of T12-L1. The region is lifted to cranial during an exhalation of the The osteopath stretches the patient and then the region is mobilised to mediolateral. region of the coeliac plexus The technique is repeated several times. several times to caudal while the patient remains bent This mobilization will improve the circulatory supply and forwards (for optimal contact) therefore the trophic condition of the region around the coeliac trunk and plexus. Any adhesions and retractions are also The osteopath holds the stretched. contact on the coeliac plexus to posterior/caudal while the patient exhales and stands up Figure 10 – Lift and mobilization of the duodenojejunal angle straight.

The effect of this technique is very strong and must be used with due consideration. If neurovegetative signs such as sweating occur, the force of the contact should be reduced. It should be clear that this technique is contraindicated in cases of arteriosclerosis. References Problems of the coeliac plexus will often result in local pain 1. Bacourt F, Goeau-Brissonnière O, Koskas F. Compression associé with palpation. Avoid this pain during the technique. du tronc coeliaque et the l’artère meséntérique supérieure par le During or just after the technique orthostatic hypotension can diaphragme. Chirurgie. 1988. 114:762-768. occur. Inform the patient that this frequently occurring reaction 2. Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW. Median will spontaneously resolve. arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. Am. J. Gastroenterol. 1997. 92:3:519-523. Defibrosing of the coeliac plexus (patient supine) 3. Carey JP, Stemmer EA, Connolly JE. Median arcuate ligament The patient is supine with both legs bent. syndrome. Arch. Surg. 1969. 99:441-446. The osteopath stands next to the patient and uses both hands to 4. Desmond CP and Roberts SK. Exercise - related abdominal pain as hook lateral/posterior around the small intestine in the direction a manifestation of the median arcuate ligament syndrome. Scand. J. of the coeliac trunk. This is in the direction of the bodies of spinal Gastroenterol. 2004. 39:12:1310 -1313. levels T12-L1. The osteopath hooks around ventrally to the spine 5. Drapanas T and Bron KM. Stenosis of the celiac artery. Ann. Surg. and mobilises towards him/herself. The technique is done during 1966. 164:1085-1088. exhalation otherwise the technique cannot be done deeply enough. 6. Dubbins PA and Scholbach T. Celiac artery compression in The technique is gentle, pain free and done several times on children, adolescents, and young adults. J. Ultrasound Med. 2006 both sides. 25:8:1108 - 1109. It should be clear that this technique is contraindicated in cases 7. Dunbar JD, Molnar W, Beman FF, Marable SA. Compression of of arteriosclerosis. the celiac trunk and abdominal angina. Am. J. Roentgenol. Radium. Ther. Nucl. Med. 1965. 95:731.

Volume 20, Issue 2, June 2010 The AAO Journal Page 17 8. Edwards AJ, Hamilton JD, Nichol WD, Taylor GW, Dawson Abdominal pain and alcohol celiac plexus nerve block. AM. Experience with Coeliac Axis Compression Syndrome. Anesth. Analg. 1977. 56:1-5. Br. Med. J. 1970. 1:5692:342–345. 25. Of Gossun M, Morobe J, Of Laethem A, Burette A, 9. Fadhli HA. Congenital diaphragmatic obstrution of the aorta Somerhausen J, and Schockert J. Syndrome the compression and the celiac artery. J. Thorac. Cardiovasc. Surg. 1968. isolée du tronc coeliaque (SCITC). À propos the 8 cas. Acta 55:431-433. Chir. Belg. 1984. 84:379-383. 10. Ghosn PB, Rabbat AG, Trudel J, D’Amico P, Lecours R, 26. Vlasova MI. Individual variability in the structure and Trudel J. Celiac compression syndrome. Can. J. Surg. 1982. topography of the celiac plexus and its clinical significance. 25:4:377-379. Morfologia. 2000. 117:1:16-19. 11. Harjola PT. A rare obstruction of the coeliac artery. Ann. 27. Warter J, Storck D, Kieny R, Tongio J. Sténoses congénitals Chir. Gynaecol. Fenn. 1963. 52:547-570. du tronc coeliaque. Arch. Fr. Mal. Appar. Dig. 1970. 59:765- 780. 12. Harjola PT. Coeliac axis constriction and abdominal angina. Bull. Soc. Intern. Chir. 1968. 27:464-467. 28. Williams S, Gillespie P, Little JM. Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery. 13. Harjola PT and Lahtiharju A. Celiac axis syndrome. 1985 98:5:879-887. Abdominal angina caused by external compression of the celiac artery. Am. J. Surg. 1968. 115:864-869. Accepted for publication: May 2010 14. Horton KM, Talamini MA, Fishman EK. Median arcuate Address correspondence to: ligament syndrome: evaluation with CT angiography. Luc Peeters, DO-MROB, BSc.Ost.Med. Radiographics. 2005. 25:1177. Klein Dokkaai 3/5 15. Jamieson CW. Coeliac axis compression syndrome. Br. Med. B-9000 Ghent J. 1986. 293:159-160. Belgium Email: [email protected] 16. Marable SA, Molnar W, Beman FM. Abdominal pain secondary to celiac axis compression. Am. J. Surg. 1966. 111:493-495. 17. Mihas AA, Laws HL, Jander HP. Surgical treatment of CME QUIZ the celiac axis compression syndrome. Am. J. Surg. 1977. 133:6:688-691. The purpose of the quiz found on page 28 is to provide a convenient means of self-assessment for your reading of the 18. Petrella S, Sousa Rodriguez CF, Sgrott EA, Medeiros scientific content in “Median Arcuate Ligament Syndrome – Fernandes GJ, Marques SR, Prates JC. Relationship of Literature Study and Osteopathic Considerations” by Luc the Celiac Trunk with Median Arcuate Ligament of the Peeters, DO-MROB, BSc.Ost.Med. Diaphragm. Int. J. Morphol. 2006. 24:2:263-274. 19. Promwikorn W, Thongpila S, Pradidarcheep W, Mingsakul T, Answer each question listed. The correct answers will be Chunhabundit P, Somana R. Angioarchitecture of the coeliac published in the September 2010 issue of the AAOJ. sympathetic ganglion complex in the common tree shrew (Tupaia glis) J. Anat. 1998. 193:3:409–416. To apply for Category 2-B CME credit, transfer your answers to the AAOJ CME quiz application form answer sheet 20. Reuter SR. Accentuation of celiac compression by the on page 28. The AAO will record the fact that you submitted median arcuate ligament of the diaphragm during deep the form for Category 2-B CME credit and will forward your expiration. Diagn. Radiol. 1971. 98:561-564. test results to the AOA Division of CME for documentation. 21. Reuter SR and Bernstein EF. The anatomic basis for You must have a 70% accuracy in order to receive CME credits. respiratory variation in median ligament compression of the celiac artery. Surgery. 1973. 73:381-385. 22. Saenko VF, Virchenko SB, Kucherenko TL and Belianskii LS. The role of Treitz’ ligament in regulating the evacuation of solid food from the duodenum. Biull. Eksp. Biol. Med. 1989. 108(9):263-266. CLASSIFIED AD LOOKING FOR A CERTIFIED DO to be an associate in 23. Sulkowski U and Wolters U. Das Syndrom des Median a busy (fee for service) practice in the northern Virginia arcuate ligament mit Zweigefäßbeteiligung. Uit the boek area. Certification in Family Practice is helpful. Please Gefässchirurgie (Ausgabe NaN/2000) Springer Verlag. 2000. contact [email protected]. 24. Thompson GE, Moore DC, Bridenbaugh LD, Artin RY.

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